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| __NOTOC__ | | __NOTOC__ |
| {{Paraganglioma}} | | {{Paraganglioma}} |
| {{CMG}} {{AE}} {{AAM}} | | {{CMG}} {{AE}} |
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| ==Overview== | | ==Overview== |
| '''Paraganglioma''' is a rare [[neoplasm]] that can be found in the abdomen, thorax, head and neck. They are usually considered [[benign]], complete surgical removal results in cure. However, in about 3% of cases they are [[malignant]] and have the ability to [[metastasis|metastasize]]. Paragangliomas are still sometimes called '''glomus tumors''' (not to be confused with [[glomus tumor]]s of the skin) and '''chemodectomas''', but paraganglioma is the currently accepted and preferred term.
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| Paragangliomas are found predominantly in the abdomen (85%, thorax (12%), and only 3% are found in the head and neck region. Most occur as single tumors. When they occur in multiple sites they are usually found as a part of a heritable syndrome, such as [[multiple endocrine neoplasia]] types II-A and II-B and '''''[[SDH]]'''''-related mutations. Symptoms of paraganglioma include [[dysphagia]], [[dizziness]], and hearing problems. MRI is helpful in localization of paraganglioma which demonstrates a high signal mass.<ref name="cancergov"> National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_94_toc</ref>
| | ==Historical Perspective== |
| Surgery is the mainstay of treatment for paraganglioma.
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| ==Historical perspective== | |
| Glomus tumor name formerly (and incorrectly) used paraganglioma.
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| ==Classification== | | ==Classification== |
| Paraganglioma may be classified into several subtypes based on the site of origin and having a neural cell line.
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| ==Pathophysiology== | | ==Pathophysiology== |
| On gross pathology, sharply circumscribed polypoid red vascular masses with the firm to rubbery in consistency are characteristic of paragangliomas. On microscopic inspection, the tumor cells are readily recognized. Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen. These cell balls are separated by fibrovascular [[stroma]] and surrounded by
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| sustentacular cells.
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| ==Causes== | | ==Causes== |
| There are no established causes for paraganglioma, approximately 25% of cases are inherited.
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| ==Differentiating Paraganglioma From Other Diseases== | | ==Differentiating Xyz from Other Diseases== |
| Paraganglioma must be differentiated from [[carcinoid tumor]], neuroendocrine carcinoma, medullary carcinoma of the thyroid, middle ear [[adenoma]], and [[meningioma]].
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| ==Epidemiology and Demographics== | | ==Epidemiology and Demographics== |
| Paraganglioma is a rare disease, familial paragangliomas account for approximately 25% of cases and occur at an earlier age.
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| ==Complication== | | ==Risk Factors== |
| Complications associated with paraganglioma may include [[Hoarseness]], [[Dysphagia]], [[Hearing loss]], and [[Facial paralysis]]. | | |
| | ==Screening== |
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| | ==Natural History, Complications, and Prognosis== |
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| ==Diagnosis== | | ==Diagnosis== |
| ===Staging=== | | ===Diagnostic Study of Choice=== |
| There is no established system for the staging of paraganglioma.
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| ===Symptoms=== | |
| Symptoms of paraganglioma include [[dysphagia]], [[dizziness]], [[tinnitus]] and [[hearing loss]].
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| ===Physical Examination Finding=== | | ===History and Symptoms=== |
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| Common physical examination findings of paragangliomas include painless neck mass, hearing loss, and red eardrum.
| | ===Physical Examination=== |
| ===CT=== | | |
| | ===Laboratory Findings=== |
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| | ===Electrocardiogram=== |
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| | ===X-ray=== |
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| | ===Echocardiography and Ultrasound=== |
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| | ===CT scan=== |
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| CT scan is helpful in localization of paraganglioma.<ref name="cancergov"> National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_94_toc</ref>
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| ===MRI=== | | ===MRI=== |
| MRI is helpful in localization of paraganglioma which demonstrates a high signal mass.<ref name="cancergov"> National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_94_toc</ref>
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| ===Other Imaging Studies=== | | ===Other Imaging Findings=== |
| <sup>123</sup>I-metaiodobenzylguanidine (MIBG) scintigraphy coupled with CT imaging can be used for diagnosis of paragnglioma.
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| ===Other Diagnostic Studies=== | | ===Other Diagnostic Studies=== |
| [[Immunohistochemistry]] and [[histochemistry]] are used in the diagnosis of paraganglioma.
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy=== | | ===Medical Therapy=== |
| Pharmacological medical therapies for paraganglioma include alpha blockers, beta blockers, and chemotherapy.
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| | === Interventions === |
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| ===Surgery=== | | ===Surgery=== |
| Surgery is the mainstay of treatment for paraganglioma.
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| | ===Primary Prevention=== |
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| | ===Secondary Prevention=== |
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| ==References== | | ==References== |
| {{reflist|2}} | | {{reflist|2}} |
| {{Epithelial neoplasms}}
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| | [[Category:Oncology]] |
| [[Category:Disease]] | | [[Category:Medicine]] |
| [[Category:Types of cancer]] | | [[Category:Neurology]] |
| [[Category:Mature chapter]] | | [[Category:Endocrinology]] |